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Motor neuron–immune interactions: the vicious circle of ALS

  • Movement Disorders - Review article
  • Published:
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Abstract

Because microglial cells, the resident macrophages of the CNS, react to any lesion of the nervous system, they have for long been regarded as potential players in the pathogenesis of several neurodegenerative disorders including amyotrophic lateral sclerosis, the most common motor neuron disease in the adult. In recent years, this microglial reaction to motor neuron injury, in particular, and the innate immune response, in general, has been implicated in the progression of the disease, in mouse models of ALS. The mechanisms by which microglial cells influence motor neuron death in ALS are still largely unknown. Microglial activation increases over the course of the disease and is associated with an alteration in the production of toxic factors and also neurotrophic factors. Adding to the microglial/macrophage response to motor neuron degeneration, the adaptive immune system can likewise influence the disease process. Exploring these motor neuron–immune interactions could lead to a better understanding in the physiopathology of ALS to find new pathways to slow down motor neuron degeneration.

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Acknowledgments

Support for S.B. comes from Institut National de la Santé et de la Recherche Medicale (INSERM), for A.B. from an Ecole des Neurosciences de Paris île-de-France (ENP)-fellowship and for P.M. from a French Research and Education Ministry fellowship. The authors would like to thank Michel Mallat and Christian S. Lobsiger for their comments on the manuscript. Research is supported by the Muscular Dystrophy Association (MDA), European FP7 Marie Curie funds, Thierry Latran foundation, INSERM and UPMC.

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  1. Centre de Recherche de l’Institut du Cerveau et de la Moelle Epinière, Institut National de la Santé et de la Recherche Médicale (INSERM), Unité Mixte de Recherche S-975, Centre National de Recherche Scientifique (CNRS) Unité Mixte de Recherche 7225, Université Pierre et Marie Curie (UPMC), Hôpital de la Salpêtrière, Bâtiment Pharmacie, 47, bd de l’hôpital, 75013, Paris, France

    Ana G. Barbeito, Pinar Mesci & Séverine Boillée

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  1. Ana G. Barbeito
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Barbeito, A.G., Mesci, P. & Boillée, S. Motor neuron–immune interactions: the vicious circle of ALS. J Neural Transm 117, 981–1000 (2010). https://doi.org/10.1007/s00702-010-0429-0

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